Short Bowel Syndrome

Objectives

Definition Etiology Clinical manifestation Management prognosis Complication

Definition It is a malabsorpative state that may

follow massive resection of small intestine.

There is no specific intestinal length at which SBS well clinically present.

The small intestine of the neonate is about 250 cm in length, 750 cm in adult.

Infants have more favorable long term prognosis.

Factors that influence the length of time until child independent of TPN

Extent/ location of resection. Presence or absence of colon Presence /Absence of ICV. Degree of adaptation in remaining bowel. Extent of residual bowel disease or

complications e.g. adhesions, strictures

Etiology

Congenital anomalies : Intestinal Atresia . Gastroschisis omphalocele Hirschsprung’s

disease,

Acquired : Resection of bowel: NEC , Crohn’s disease volulus, tumor , radiation enteritis, ischemic injury

Manifestation

Fluid & electlytes imbalance.

Steatorrhea Wt loss and malnutrition. Minerals def: Ca, Mg, Iron,

zinc, B12, fat soluble vit. Malabsorption of CHO and

protein.

Metabolic acidosis. Gastric acid hypersecretion. Cholelithiasis. Liver disease, cholestasis. Bone disease. Complications related to

TPN.

Reduction of functioning bowel mass to below min necessary to balance supply & demand of essential body needs leading to intestinal failure manifested as:

Manifestation related to site of resection

Duodenal resection Jejunal resection Ileal resection Loss of the ileocecal valve Colon

Duodenal resection

Protein , CHO, fat maldigestion Ca, mg, iron, folate malabsorption Fat soluble vit deficiency

Jejunal resection CHO Malabsorption. Water soluble vit defiency . Malabsorption is transient (ileal

adaptation).

Ileal resection Steatorrhea as bile salts not absorbed. Cholesterol stones secondary to loss of

bile acids. Fat soluble vit def. B12 def Loss of ileal brake, decrease transit time

causing diarrhea.

Loss of the ileocecal valve

Bacterial overgrowth: allows bacteria to flux into ilium

Rapid transit time that exacerbate malabsorption.

Colon Role of The Colon: water absorption. It gives additional length, it slows transit time

and slows gasteric empting, But

Deconjugation of bile acids by colonic bacteria & secondary secretory diarrhea.

lactic acidosis: conversion of CHO by lactobacillus to D-lactic acid lead to high AG metabolic acidosis,

Intestinal adaptation Starts 24-48 hrs post-op, (enteral feeds

as early as possible). Lasts up to 11-12 years . Change in morphorogy and functional

capacity.

Change in morphorogy

Macroscopic Increase in length

Microscopic Villus: increase height and diameter Crypt: elongation Epithelial cell life cycle: increase proliferation decrease apoptosis.

Change in functional capacity

Increase absorption per unit length Upregulation of sodium glucose

transporter.

Lab investigation

Blood U&E, bone profile, & mg, PRN then

biweekly CBC, triglycerides, cholesterol Weekly Folate, vit B12, copper, zinc, Monthly Blood gas and AG for suspected lactic

acidosis.

Microbiology

If sepsis suspected; blood & urine c/s Cultures from both the central and

peripheral sites. Consider opportunistic infections, so

search for fungal infection.

Imaging Studies

To assess for potential complications, Infection

Abdominal ultrasonography to look for fungal balls in the kidney

Bowel obstruction Plain radiography. Barium imaging of the bowel

Liver disease Abdominal US to study the liver, biliary tract,

& presence of ascites.

Management During early period after intestinal

resection, TPN to prevent fluid and electrolytes imblance.

Stomal & fecal losses replaced q 2 hrs with solution separate from TPN.

May develop gastric hypersecretion so give H2 blocker

Management

The goals of nutritional therapy 1.Maintain adequate nutrition 2.Promote intestinal adaptation 3.Avoid complications

TPN for the first 7-10 days TPN :30 kcal/kg/day Enteral feeding when hemodynamic

stable and fluid management stable. Continuous enteral feeds: to prevent

osmotic diarrhea. Bolus feeds less well tolerated. Formula osmolality should be < 310

mosm/kg.

Composition

Protien hydrolysate or elemental diets Complex carbohydrate is better than

simple carbohydrate Oxalate restriction in patient with an

intact colon and fat malabsorption to avoid stone formation.

Lipid Medium-chain triglycerides

Better absorbed in the presence of bile acid or pancreatic insufficiency.

Long-chain triglycerides : more effective in stimulating intestinal adaptation

Mix MCT + LCT

Indications for continued parental nutrition Poor weight gain or loss of maintenance

weight. Extensive stomal fluid and electrolyte

losses which cannot be replaced orally.

Pharmacologic therapy

Decrease stomal secretory losses H2 blockers, PPI & octreotide ??loperamide

Ursodeoxycholic acid: Improves bile acid–dependent bile flow.

Antibiotics used to prevent small-bowel overgrowth. Insufficient data regarding -glutamine affects clinical

outcomes in infants. GH in children with SBS may have some benefit in

those with low or limited GH responsiveness.

Surgical Care

Surgical care is related to venous access (ie, central line placement to provide TPN).

Gastrostomy tube placement to provide for enteral access.

Nontransplantation procedures To improve the surface area or to slow transit

emptying time. Bianchi procedure (intestinal tapering or

lengthening) Indicated in small bowel with bacterial

overgrowth ,dilated bowel and continued malabsorption

Cutting bowel longitudinally, and create a segment of bowel twice length, half diameter without loss of mucosal surface area.

TaperingTapering Bowel lengtheningBowel lengthening

Indications Impending or overt liver failure IV access loss Frequent central line related sepsis Intestinal failure

Small bowel transplantation

Prognosis

Ultimately patient with SBS may be successfully wean from TPN although the entire process may take several years.

Intestinal transplantation should be consider as a last resort.

Complications

Early complications Catheter related

complication

chronic complications liver & biliary disease Bacterial overgrowth D-lactic acidosis, Nutritional def,

Bacterial overgrowth Defined as increased bacterial content in the

small intestine Occurs if no Ileum , dilated bowel loops with

hypomotility segment & strictures. Clinically: N, V, distension, FTT, increase

hepatic injury from TPN , GI blood loss Also common cause of clinical deterioration in

a previously stable patient with SBS. Diagnosed duodenal fluid analysis, culture,

stool c/s, H2 breath test.

This well leads to CHO malabsorption, worsening of osmotic diarrhea, and increased risk of metabolic acidosis and dehydration.

Treatment with antibiotic , including administration of metronidazole alternating with oral gentamicin.

Should be cycled on a weekly or biweekly basis.

Conclusion Early management of SBS replacement of fluid

and electrolytes. Enteral feeding should begin once the patient

stabilizes. Continuous enteral feeding or is preferred. For enteral feedings, hypoallergenic protein

hydrolysate formulas or breast milk are usually best tolerated

Several pharmacological approaches have been tested to enhance intestinal adaptation and improve feeding tolerance. None of these approaches are proven to be helpful, but studies are ongoing.

References

1- www.uptodate.com. Management of the short bowel syndrome in children , September 2009

2- www.emdicine .com , Short Bowel Syndrome, Carmen .C. Apr 2009

3- Dr. Siham

Thanks